Quick Links
About MyLymphomaTeam
Powered By
Did you know that several different types of cancer can affect the small intestines? Doctors classify these cancers based on the type of cells involved. About 15 percent to 20 percent of intestinal cancers are lymphomas. According to BMJ Open Gastroenterology, intestinal lymphomas usually affect adults aged 60 and older, developing slightly more often in men than women.
Intestinal lymphoma is rare but treatable, especially when detected early. In this article, we’ll cover its symptoms, diagnosis, treatment options, and prognosis (outlook). If you feel uncertain about what to do next, understanding the condition can empower you to make the best decisions for your health.
Lymphoma is a cancer that develops from lymphocytes (a type of white blood cell). There are two main types of lymphoma: Hodgkin lymphoma and non-Hodgkin lymphoma (NHL).
The lymphatic system, which helps the body fight infection and remove waste, is often affected by lymphoma. Lymphoma causes an abnormal buildup of lymphocytes in the lymph nodes, such as those in the neck, armpits, or groin. Sometimes, lymphoma develops in extranodal sites (areas outside the lymph nodes). When it begins in these areas, it’s considered primary extranodal lymphoma.
About 30 percent to 40 percent of extranodal lymphomas develop in the gastrointestinal (GI) tract (digestive system). Almost all GI lymphomas are a type of non-Hodgkin lymphoma. Most GI lymphomas affect the stomach — these are called primary gastrointestinal lymphomas. The small intestines, also called the small bowels, are the second most common spot for GI lymphomas to develop. Lymphoma can develop in any part of the small intestine: the duodenum (the first section that receives food from the stomach), the jejunum (the middle section where nutrients are absorbed), or the ileum (the final section that connects to the large intestine). Gastrointestinal lymphomas may also affect the throat, esophagus (the tube that connects the mouth to the stomach), colon (large intestine), and rectum.
Beyond the main types of intestinal lymphoma — Hodgkin lymphoma and non-Hodgkin lymphoma — the disease is divided into subtypes based on the cells they affect. About 9 out of 10 GI lymphomas affect a type of lymphocyte called B cells. GI lymphomas rarely affect the T cells (another type of lymphocyte). Intestinal B-cell lymphomas include:
There are two kinds of intestinal T-cell lymphomas. The first is called enteropathy-associated T-cell lymphoma (EATL), which means it’s linked to diseases of the intestine. It occurs only in people who have celiac disease and was previously called EATL type 1.
The second is called monomorphic epitheliotropic intestinal T-cell lymphoma, which starts in the T cells of the small intestine. Researchers say this kind of lymphoma — previously called EATL type 2 — is more likely to develop in people who are Asian or Hispanic.
Intestinal lymphoma often causes gastric symptoms — problems that originate in the gastrointestinal tract. These can include:
T-cell intestinal lymphoma may also cause an itchy rash. It sometimes leads to B symptoms, which include fevers, night sweats, and a loss of more than 10 percent of a person’s body weight.
Intestinal lymphoma can cause other serious medical problems. If the tumor grows, it can block the intestinal lumen, the hollow space inside the intestines where food passes through during digestion.
In one study of more than 1,000 people with the condition, nearly 1 out of 9 people developed a hole (perforation) in their GI tract. Intestinal perforations are a medical emergency. These holes can lead to fever, chills, extreme abdominal pain, nausea, and vomiting. Perforations can be caused by the lymphoma itself or by its treatments.
If your doctor suspects that you have gastric (intestinal) problems, they will recommend several tests. This can help determine if you have intestinal lymphoma or something else, like inflammatory bowel disease. The tests may include:
Intestinal lymphomas are also diagnosed using endoscopy. During an endoscopic procedure, a doctor passes a thin tube containing a camera through your mouth or your rectum. Another approach is to swallow a small pill-sized capsule that contains a camera. The camera collects images that can offer a closer look at intestinal tissue. Doctors may take a biopsy (remove a small piece of tissue) during the endoscopy. Then, they carefully study the sample under a microscope and look for lymphoma cells.
An important part of diagnosis is determining the stage of the disease. In lymphoma, staging helps doctors estimate someone’s outlook and know which treatments may be best. Doctors may determine the lymphoma stage using one of three staging systems. These include:
Each of these systems uses slightly different methods to describe what areas of the body contain lymphoma cells. To stage GI lymphoma, doctors use imaging tests like:
Different subtypes of intestinal lymphoma are treated in different ways. People without any symptoms or with indolent (slow-growing) lymphoma may not need treatment right away. They may be able to wait until the disease gets worse and symptoms appear.
Surgery can help doctors diagnose and treat intestinal lymphoma. Surgery used to be a more common treatment for intestinal lymphoma. However, as lymphoma medications have improved, doctors use surgery less frequently than they once did. Now, surgery is often only used to treat problems such as perforation. The procedure used to remove part of the damaged intestine is called a resection.
Oncology (cancer) specialists use chemotherapy regimens (combinations of different drugs) to treat intestinal lymphoma. Possible regimens include:
Doctors sometimes combine chemotherapy with newer drugs like rituximab or polatuzumab vedotin (Polivy) to treat certain types of B-cell lymphomas more effectively. Recently approved drugs like loncastuximab tesirine (Zynlonta) are also being used for some cases of lymphoma.
Sometimes, lymphoma doesn’t go away after treatment. This is called refractory lymphoma. Other times, it comes back after disappearing (relapsed lymphoma). Doctors often treat these cases with different chemotherapy regimens. For example, one of these is called DHAP. It includes dexamethasone (sold as Decadron), high-dose cytarabine (also called ara-C, both sold as Cytosar-U), and cisplatin (formerly sold as Platinol).
Chimeric antigen receptor (CAR) T-cell therapy is a specialized type of immunotherapy. It works by genetically modifying damaged T cells with targeted drugs to boost the immune system and destroy cancer cells.
This therapy has shown promising results in treating relapsed or refractory cases of intestinal lymphoma and aggressive non-Hodgkin lymphomas. CAR T-cell therapies for certain types of lymphoma include:
CAR T-cell therapy is rapidly advancing, with new treatments for different types of lymphoma being approved frequently.
CAR T-cell therapy involves several steps. First, T cells are collected from your blood in a process called leukapheresis. These cells are sent to a lab, where they’re modified to target cancer cells and grow in large numbers. Before receiving the CAR T cells, you get a short round of chemotherapy to prepare your body. Then, the modified T cells are infused back into you like a blood transfusion. Most people will spend one to two weeks in the hospital afterward to monitor for side effects.
People with intestinal T-cell lymphoma often live longer and have more time before a relapse when they use a treatment plan called the Newcastle regimen, or IVE/MTX-ASCT. This treatment combines a stem cell transplant with the chemotherapy drugs ifosfamide (sold as Ifex), etoposide, epirubicin (sold as Ellence), and methotrexate (sold as Rheumatrex and Trexall).
Stem cell transplantation is a procedure in which your unhealthy stem cells (the cells that make new blood cells) are replaced with healthy ones. Intestinal lymphoma is generally treated with an autologous (self) stem cell transplant. During this procedure, doctors remove your stem cells before giving you high doses of chemotherapy or radiation therapy. The aggressive treatment kills many of your lymphoma cells as well as your healthy blood cells. Once the treatment is done, doctors place the “rescued” autologous stem cells back into your body. Those healthy stem cells then create new blood cells.
In some cases, intestinal lymphoma may be treated with an allogeneic (donor) stem cell transplant. This procedure is similar to an autologous transplant, but the new stem cells come from a donor.
Targeted therapies are drugs that recognize and block specific molecules found in cancer cells. Rituxan (rituximab) is one targeted therapy sometimes given along with chemotherapy to treat intestinal B-cell lymphoma.
Newer types of targeted therapy drugs include monoclonal antibody therapy, which uses lab-made antibodies (proteins used by the immune system) to attack and destroy cancer cells.
Your prognosis depends on the stage at diagnosis, subtype, and overall health. People with early-stage lymphoma generally have better outcomes than those who are diagnosed with late-stage disease. Also, different gastric lymphoma subtypes have different overall survival rates and relapse rates.
Advances in new testing methods now help predict outcomes and tailor treatments more effectively. For example, molecular profiling looks at the genetic and molecular “blueprint” of the cancer cells to find specific mutations (changes). This helps doctors pick treatments that are most likely to work for that specific type of cancer.
Minimal residual disease testing is used to find tiny amounts of cancer cells that might still be in the body after treatment. This can help doctors decide if more treatment is needed to prevent the cancer from coming back.
Studies show that 5-year survival rates (the number of people still alive five years or more after being diagnosed and treated) are higher than 70 percent for some B-cell subtypes diagnosed at stages 1 or 2. People with advanced subtypes, such as T-cell lymphomas, often have lower survival rates.
Doctors determine intestinal lymphoma prognosis using the International Prognostic Index (IPI). It provides a score that describes a person’s risk level. People with a high-risk score are more likely to have worse outcomes than those with a low-risk score. An IPI score is based on:
Many cases of intestinal lymphoma are treatable, especially if caught early. Localized disease affects only a specific part of the body and hasn’t spread elsewhere. For localized disease, cure rates are higher with a combination of chemotherapy, targeted therapy, or surgery. However, people with advanced or aggressive subtypes may need more intensive treatments. Unfortunately, lymphoma can come back even if it’s been treated successfully before. Always follow up with your doctor if you experience any new or worsening symptoms.
On MyLymphomaTeam, the social network for people with lymphoma and their loved ones, more than 21,000 members come together to ask questions, give advice, and share their stories with others who understand life with lymphoma.
Are you living with intestinal lymphoma? Share your experiences in the comments below, or start a conversation by posting on your Activities page.
Fatima Sharif, MBBS, FCPS
graduated from Aga Khan University, Pakistan, in 2017 after completing medical school.
Learn more about her here.
Maureen McNulty
studied molecular genetics and English at Ohio State University.
Learn more about her here.
Zoe Owrutsky, Ph.D.
earned her Bachelor of Science from the University of Pittsburgh in 2014 and her Ph.D. in neuroscience from the University of Colorado Anschutz Medical Campus in 2023.
Learn more about her here.
Get updates directly to your inbox.
Sign up for free!
Continue with Facebook
Sign up with your email
Become a member to get even more
A MyLymphomaTeam Member
So glad to have come across this article as there seems to be so little written about the condition.
We'd love to hear from you! Please share your name and email to post and read comments.
You'll also get the latest articles directly to your inbox.